La mastocitosi sistemica indolente e il trattamento con la fototerapia con UVB a banda stretta: studio di 4 casi

摘要: Mastocytoses represent a heterogeneous group of stem cell disorders marked by an abnormal hyperplasia and accumulation mast cells (MC) in one or more tissues, including bone marrow, gastrointestinal tract, liver, spleen, lymph nodes skin. Indolent systemic mastocytosis (ISM) is the most frequently diagnosed variant characterized red-brownish pruriginous maculopapular lesions, marrow infiltration (multifocal dense infiltrates cells) without functional impairment indolent clinical course with good prognosis. The main symptoms are related to tissue response release mediators from local burden, mild-high pruritus being far common cutaneous symptom. Patients ISM treated mediator-tergeting drugs anti-histamine, but not cytoreductive agents. We present four cases prevalent symptomatology, deficient anti-histamines intolerable contraindications employ PUVA-therapy, therefore narrowband UVB phototherapy (NB-UVB). Our work suggests for first time that NB-UVB useful as second-line therapeutic modality treatment patients whom PUVA-therapy has failed cannot be administered antihistamine provide inadequate control symptomatology.